Familial hypertrophic cardiomyopathy (FHC) is an inherited autosomal trait that is most commonly caused by mutations in sarcomeric protein genes. Of the 10 different sarcomeric genes identified to cause hypertrophic cardiomyopathy (HCM), mutations in cardiac myosin binding protein C (cMyBPC) are among the most common of all known hereditary linked HCM. FHC impacts approximately 1 in 500 individuals; however, several founding mutations in cMyBPC in different regions of the world have been recently shown to affect disproportionally large numbers of families from common ancestry who are at significantly greater risk for development of cardiac dysfunction and heart failure compared to the individual who do not carry these mutations. In the last 15 years, approximately 200 disease causing cMyBPC mutations have been identified, which have been associated with variable degrees of contractile dysfunction and hypertrophy. The majorities of cMyBPC mutations are heterozygous and have variable clinical presentation, but homozygous mutations are associated with severe hypertrophy and high mortality rates at a young age.